AA Amyloidosis in the Course of HIV Infection: A Report of 19 Cases Including 4 New French Cases and a Comprehensive Review of Literature

Nephron ◽  
2021 ◽  
pp. 1-9
Author(s):  
Paul Breillat ◽  
Valérie Pourcher ◽  
Samuel Deshayes ◽  
David Buob ◽  
Alexandre Cez ◽  
...  
Keyword(s):  
Hiv Infection ◽  
Renal Involvement ◽  
Significant Risk ◽  
Rare Condition ◽  
Aa Amyloidosis ◽  
Inflammatory Conditions ◽  
Risk Of Progression ◽  
Stage Renal Disease ◽  
End Stage ◽  
National Reference Center

<b><i>Introduction:</i></b> HIV infection has been recently retained as an unclear cause of AA amyloidosis. Our aim was to investigate cases of AA amyloidosis associated with HIV infection to understand if it could be considered as a cause of AA amyloidosis. <b><i>Methods:</i></b> A comprehensive literature review was conducted as well as retrospective study from French cases collected from our national reference center for AA amyloidosis. <b><i>Results:</i></b> Altogether, 19 patients with AA amyloidosis and HIV infection were found with 68% of men and median age at amyloidosis diagnosis of 38 years (range 28–75 years). Clinical presentation was nephrotic syndrome in 94% (<i>n</i> = 17/18). Among patients with renal involvement and assessable outcome (<i>n</i> = 17), 11 (64.7%) progressed to chronic kidney disease, with 6 (35%) end-stage renal disease. Seventy-five percent of patients had uncontrolled HIV infection and 71.4% CD4 counts &#x3c;400/mm<sup>3</sup> at amyloidosis diagnosis. Repeated or chronic bacterial or fungal infection was found in 47% of cases and a history of parenteral drug use in 55% of patients. Three patients had no classical or at least no suspected AA amyloidosis cause found or reported. <b><i>Conclusions:</i></b> AA Amyloidosis is a rare condition in HIV patients with common renal involvement and significant risk of progression to chronic renal insufficiency. Because of the frequency related to other inflammatory conditions in this population, HIV is probably not an independent risk factor for AA amyloidosis.

scholarly journals AB0820 INFLUENCE OF ANCA ANTIBODIES ON DEMOGRAPHIC AND CLINICAL CHARACTERISTIC OF AAV

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1434.1-1434
Author(s):  
K. Wójcik ◽  
A. Masiak ◽  
Z. Zdrojewski ◽  
R. Jeleniewicz ◽  
M. Majdan ◽  
...  
Keyword(s):  
Renal Disease ◽  
End Stage Renal Disease ◽  
Clinical Characteristics ◽  
Renal Involvement ◽  
Organ Involvement ◽  
Unknown Etiology ◽  
Anca Associated Vasculitis ◽  
Stage Renal Disease ◽  
End Stage ◽  
Male To Female

Background:ANCA associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown etiology and the broad clinical spectrum ranging from life-threatening systemic disease, through single organ involvement to minor isolated skin changes. Unfortunately the clinical classification, ANCA specificity or genetic characteristics alone is not able to categorize AAV patients in a satisfactory manner. As a consequence advanced statistical techniques were used to identify and stratify AAV subphenotypes [1, 2]. Here we have analyzed influence of the ANCA type on clinical manifestations and demographic characteristics in various types of AAV, based on data from the POLVAS registryObjectives:We decided to retrospectively analyze a large cohort of Polish AAV patients deriving from several referral centers – members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS) – and concentrate on demographic and clinical characteristics of anti-PR3 and anti-MPO positive patients regardless of their clinical diagnosis.Methods:We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016. Patients were enrolled by 9 referral centers. We analyzed dichotomous variables: gender; ANCA status – anti-PR3+ or anti-MPO+, ANCA negative; organ involvement - skin, eye, ENT, respiratory, heart, GI, renal, urinary, CNS, peripheral nerves and polytomous variable (number of relapses), supported by quantitative covariates (e.g., age at diagnosis, CRP at diagnosis, maximal serum creatinine concentration ever)[3].Results:MPO-positive patients (both GPA and EGPA phenotype) were older at the time of diagnosis with a substantial percentage diagnosed > 65 years of age, and with high rate of renal involvement. Interestingly, while in the whole group of patients diagnosed with EGPA male to female ratio was 1:2, the MPO+ EGPA patients showed M:F ratio of 1:1.The analysis of ANCA negative AAV reveled significant differences in GPA, ANCA negative group is characterized with significantly lower frequency of renal involvement compared to rest GPA (11,5% vs 63,7%) p<0,05 what should be emphasized ANCA negative AAV never lead to ESRD (end stage renal disease) or even transient dialysis.Conclusion:ANCA specificity is indispensable as a separate variable in any clinically relevant analysis of AAV subcategories. MPO+ group is characterized by older age at time of diagnosis, male to female ration 1:1, kidney involvement, and shows more homogenous clinical phenotype than PR3+ AAV patients. In our group ANCA negative AAV never lead to ESRD (end stage renal disease) or even transient dialysis.References:[1]Mahr A, Specks U, Jayne D. Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum. Rheumatol Oxf Engl 2019;58:1707–9. https://doi.org/10.1093/rheumatology/kez148.[2]Wójcik K, Biedroń G, Wawrzycka-Adamczyk K, Bazan-Socha S, Ćmiel A, Zdrojewski Z et al. Subphenotypes of ANCA-associated vasculitis identified by latent class analysis. Clin Exp Rheumatol. 2020 Sep 1. Epub PMID: 32896241.[3]Wójcik K, Wawrzycka-Adamczyk K, Włudarczyk A, Sznajd J, Zdrojewski Z, Masiak A, et al. Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry. Clinical Rheumatology. 1 wrzesień 2019;38(9):2553–63.Disclosure of Interests:None declared

scholarly journals Vasculitis ANCA: Alternativas de tratamiento y las expectativas en los pacientes

2021 ◽  
pp. 1-2
Author(s):  
Carolina Aguilar-Martínez 
Keyword(s):  
Systematic Review ◽  
Cox Regression ◽  
Meta Analysis ◽  
Significant Risk ◽  
Limiting Factor ◽  
Significant Survival ◽  
Stage Renal Disease ◽  
End Stage ◽  
Immunosuppression Therapy

<b>Background:</b> The benefits of treating anti-neutrophil cytoplasmic autoantibody-associated vasculitis (AAV) in advancing age remains unclear with most published studies defining elderly as ≥65 years. This study aims to determine outcomes of induction immunosuppression in patients aged ≥75 years. <b>Methods:</b> A cohort of patients aged ≥75 years with a diagnosis of AAV between 2006 and 2018 was constructed from 2 centres. Follow-up was to 2 years or death. Analysis included multivariable Cox regression to compare mortality and end-stage renal disease (ESRD) based on receipt of induction immunosuppression therapy with either cyclophosphamide or rituximab. A systematic review of outcome studies was subsequently undertaken amongst this patient group through Pubmed, Cochrane and Embase databases from inception until October 16, 2019. <b>Results:</b> Sixty-seven patients were identified. Mean age was 79 ± 2.9 years and 82% (<i>n</i> = 55) received induction immunosuppression. Following systematic review, 4 studies were eligible for inclusion, yielding a combined total of 290 patients inclusive of our cohort. The aggregated 1-year mortality irrespective of treatment was 31% (95% CI 25–36%). Within our cohort, induction immunosuppression therapy was associated with a significantly lower 2-year mortality risk (hazard ratio [HR] 0.29 [95% CI 0.09–0.93]). The pooled HR by meta-analysis confirmed this with a significant risk reduction for death (HR 0.31 [95% CI 0.16–0.57], <i>I</i><sup>2</sup> = 0%). Treated patients had a lower pooled rate of ESRD, but was not statistically significant (HR 0.71 [95% CI 0.15–3.35]). <b>Conclusion:</b> This meta-analysis suggests that patients ≥75 years with AAV do benefit from induction immunosuppression with a significant survival benefit. Age alone should not be a limiting factor when considering treatment.

scholarly journals Low Birth Weight and Risk of Progression to End Stage Renal Disease in IgA Nephropathy—A Retrospective Registry-Based Cohort Study

PLoS ONE ◽  
2016 ◽  
Vol 11 (4) ◽  
pp. e0153819 ◽  
Author(s):  
Paschal Ruggajo ◽  
Einar Svarstad ◽  
Sabine Leh ◽  
Hans-Peter Marti ◽  
Anna Varberg Reisæther ◽  
...  
Keyword(s):  
Cohort Study ◽  
Birth Weight ◽  
Low Birth Weight ◽  
Renal Disease ◽  
Iga Nephropathy ◽  
End Stage Renal Disease ◽  
Risk Of Progression ◽  
Stage Renal Disease ◽  
End Stage

scholarly journals Neutrophils associate with Bowman’s capsule rupture specifically in PR3-ANCA glomerulonephritis

2021 ◽  
Author(s):  
Samy Hakroush ◽  
Björn Tampe
Keyword(s):  
Plasma Cells ◽  
Immune Cell ◽  
Renal Involvement ◽  
Kidney Injury ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Stage Renal Disease ◽  
End Stage ◽  
Bowman's Capsule ◽  
Tubulointerstitial Inflammation ◽  
Capsule Rupture

Abstract Background Renal involvement is a common and severe complication of ANCA (antineutrophil cytoplasmic antibody) associated vasculitis (AAV) potentially resulting in a pauci-immune necrotizing and crescentic antineutrophil cytoplasmic antibody (ANCA) glomerulonephritis (GN) with acute kidney injury (AKI), end-stage renal disease (ESRD) or death. We recently described that Bowman’s capsule rupture links glomerular damage to tubulointerstitial inflammation in ANCA-associated glomerulonephritis. Herein we provide a comprehensive histological subtyping of immune cell infiltrates in association with Bowman’s capsule rupture in ANCA GN. Methods A total of 44 kidney biopsies with ANCA GN were retrospectively included in a single-center observational study. Within a renal biopsy specimen, each glomerulus was scored separately for the presence of extensive and focal Bowman’s capsule rupture in injured glomeruli. Infiltrates of neutrophils, eosinophils, plasma cells, and mononucleated cells (macrophages, lymphocytes) were quantified as a fraction of the area of total cortical inflammation. Results Extensive Bowman’s capsule rupture was associated with tubulointerstitial inflammation containing infiltrates of neutrophils, eosinophils and plasma cells. A similar association was observed for the presence of focal Bowman’s capsule rupture, correlating with tubulointerstitial inflammation containing neutrophils, eosinophils and plasma cells. Multiple logistic regression confirmed that extensive Bowman’s capsule rupture correlated with tubulointerstitial inflammation containing neutrophils, and focal Bowman’s capsule rupture correlated with neutrophil and plasma cell infiltration. Furthermore, this association was specifically observed in PR3-ANCA GN. Conclusion To our knowledge, this is the first report linking Bowman’s capsule rupture directly to tubulointerstitial inflammation by immune cell subtypes. This underscores a pathomechanistic link between tubulointerstitial and glomerular lesions in ANCA GN and needs further investigation. Graphical abstract

scholarly journals Use of the Kidney Failure Risk Equation to Determine the Risk of Progression to End-stage Renal Disease in Children With Chronic Kidney Disease

2018 ◽  
Vol 172 (2) ◽  
pp. 174 ◽  
Author(s):  
Erica Winnicki ◽  
Charles E. McCulloch ◽  
Mark M. Mitsnefes ◽  
Susan L. Furth ◽  
Bradley A. Warady ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Renal Disease ◽  
Kidney Failure ◽  
End Stage Renal Disease ◽  
Risk Equation ◽  
Failure Risk ◽  
Risk Of Progression ◽  
Stage Renal Disease ◽  
End Stage

scholarly journals Intracardiac fistula: an unusual complication of catheter-related blood stream infection

JRSM Open ◽  
2017 ◽  
Vol 8 (9) ◽  
pp. 205427041772823 ◽  
Author(s):  
Abhilash Koratala ◽  
Hussain Aboud ◽  
Robert Gibson ◽  
Karen K Hamilton
Keyword(s):  
End Stage Renal Disease ◽  
Blood Stream Infection ◽  
Significant Risk ◽  
Blood Stream ◽  
Unusual Complication ◽  
Early Use ◽  
Venous Fistula ◽  
Stage Renal Disease ◽  
End Stage ◽  
High Index Of Suspicion

Lesson In end stage renal disease patients on dialysis, the use of catheter as a vascular access is associated with a significant risk of sepsis compared to an arterio-venous fistula. Our case emphasizes the importance of having high index of suspicion for unusual complications in patients presenting with possible catheter-related blood stream infection and early use of complementary tools such as trans-oesophageal echocardiography whenever applicable.

Acute generalised exanthematous pustulosis associated with shock

2020 ◽  
Vol 13 (10) ◽  
pp. e235846
Author(s):  
Philippos Apolinario Costa ◽  
Bruna Menon Loureiro Apolinario Costa ◽  
Clara Milikowski ◽  
Joan E St Onge
Keyword(s):  
Drug Exposure ◽  
Rare Condition ◽  
Erythematous Rash ◽  
Remarkable Improvement ◽  
Systemic Corticosteroids ◽  
History Of ◽  
Stage Renal Disease ◽  
End Stage ◽  
Exanthematous Pustulosis ◽  
Infectious Aetiology

A 23-year-old man with a history of end-stage renal disease was admitted to the hospital due to fever and shock, which occurred during his dialysis. One week prior, he developed an erythematous rash on his chest, face and back, associated with generalised eruption of pustules. In hospital, his status did not improve with norepinephrine and empirical broad-spectrum antibiotics. Following this, methylprednisolone was administered with remarkable improvement. Cultures revealed no infectious aetiology. Based on the morphology of the rash and a compatible skin biopsy, the diagnosis of acute generalised exanthematous pustulosis (AGEP) was established and considered the cause of his shock. The causative agent of his AGEP remained unknown. AGEP is a rare condition, most frequently associated with drug exposure. The removal of the offending agent is the treatment of choice. It can be complicated by shock in rare cases. In that scenario, systemic corticosteroids seem to improve outcomes greatly.

scholarly journals Kidney Disease in HIV Infection

2019 ◽  
Vol 8 (8) ◽  
pp. 1254 ◽  
Author(s):  
Gaetano Alfano ◽  
Gianni Cappelli ◽  
Francesco Fontana ◽  
Luca Di Lullo ◽  
Biagio Di Iorio ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Life Expectancy ◽  
Early Recognition ◽  
Renal Involvement ◽  
People Living With Hiv ◽  
Risk Of Death ◽  
Age Related ◽  
Stage Renal Disease ◽  
End Stage ◽  
Living With Hiv

Antiretroviral therapy (ART) has significantly improved life expectancy of infected subjects, generating a new epidemiological setting of people aging withHuman Immunodeficiency Virus (HIV). People living with HIV (PLWH), having longer life expectancy, now face several age-related conditions as well as side effects of long-term exposure of ART. Chronic kidney disease (CKD) is a common comorbidity in this population. CKD is a relentlessly progressive disease that may evolve toward end-stage renal disease (ESRD) and significantly affect quality of life and risk of death. Herein, we review current understanding of renal involvement in PLWH, mechanisms and risk factors for CKD as well as strategies for early recognition of renal dysfunction and best care of CKD.

scholarly journals Novel differences in renal gene expression in a diet-induced obesity model

2018 ◽  
Vol 314 (4) ◽  
pp. F517-F530 ◽  
Author(s):  
Victoria L. Halperin Kuhns ◽  
Jennifer L. Pluznick
Keyword(s):  
Renal Cortex ◽  
Significant Risk ◽  
Significant Risk Factor ◽  
Differentially Expressed ◽  
Diet Induced Obesity ◽  
Transcriptional Changes ◽  
Stage Renal Disease ◽  
End Stage ◽  
Upregulated Genes

Obesity is a significant risk factor for both chronic kidney disease and end-stage renal disease. To better understand disease development, we sought to identify novel genes differentially expressed early in disease progression. We first confirmed that mice fed a high-fat (HF) diet exhibit early signs of renal injury including hyperfiltration. We then performed RNA-Seq using renal cortex RNA from C57BL6/J male mice fed either HF or control (Ctrl) diet. We identified 1,134 genes differentially expressed in the cortex on HF vs. Ctrl, of which 31 genes were selected for follow-up analysis. This included the 9 most upregulated, the 11 most downregulated, and 11 genes of interest (primarily sensory receptors and G proteins). Quantitative (q)RT-PCR for these 31 genes was performed on additional male renal cortex and medulla samples, and 11 genes (including all 9 upregulated genes) were selected for further study based on qRT-PCR. We then examined expression of these 11 genes in Ctrl and HF male heart and liver samples, which demonstrated that these changes are relatively specific to the renal cortex. These 11 genes were also examined in female renal cortex, where we found that the expression changes seen in males on a HF diet are not replicated in females, even when the females are started on the diet sooner to match weight gain of the males. In sum, these data demonstrate that in a HF-diet model of early disease, novel transcriptional changes occur that are both sex specific and specific to the renal cortex.

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